The interplay of psychosis and non-compliance with fatal outcome in an adult with MSUD.

Significant progress has been achieved in enhancing early outcomes for individuals with maple syrup urine disease (MSUD), a rare metabolic disorder that leads to the accumulation of branched-chain amino acids leucine, isoleucine, and valine, where leucine is known as the primary neurotoxic metabolite. Newborn screening is helpful in early diagnosis and implementation of dietary treatment, thus reducing neurological deterioration and complications in young children. However, patients face the life-long challenge of maintaining metabolic control through adherence to a strict low-leucine diet to avoid long-term consequences of chronic hyperleucinemia, which include cognitive deficits, mood disorders, and movement disorders. This case report exemplifies the complex involvement of MSUD in adult survivors. Despite presenting early in life, the patient thrived until the onset of psychiatric symptoms. The subject of this case is a 25-year-old woman with MSUD, who remained in her usual state of health until presentation to the emergency department (ED) with psychosis and altered mental status. However, due to a lack of medical records and poor communication, there was a delay in considering MSUD as a primary cause of her psychiatric symptoms. Although a genetics consultation was later arranged and efforts were made to decrease plasma leucine to the therapeutic range, these interventions proved inadequate in halting her deterioration in health. Her condition worsened within 72 h, culminating in her untimely death. This case emphasizes the comorbidity of psychiatric involvement in MSUD, which contributes to metabolic decompensation that can lead to cerebral edema and death. This case also highlights the pressing need for enhanced strategies for the acute management and long-term care of MSUD patients with psychiatric involvement, particularly in scenarios where mental disturbance could lead to noncompliance.

Medical Aid in Dying: How Might U.S. Policy Prevent Suffering at the End of Life?

Medical aid in dying refers to policies that allow terminally ill patients to seek assistance from their medical providers to obtain medications to hasten death. In this paper, we used a 7-point policy analysis model to examine the diversity of medical aid in dying policies that exist in jurisdictions (states and territories) in the U.S. and the implications of these policies for suffering at end of life. As of this writing, these practices are available in 12 jurisdictions, including 11 states and the District of Columbia. Legalization has occurred via statutory approaches in 10 jurisdictions and permissive approaches in two jurisdictions. We discuss the structures of these laws, the requirements for participation, and the limitations created by having jurisdiction-specific policies on such broad, bioethical issues. Existing laws appear to relieve suffering in patients at end of life. Jurisdictional differences create barriers to comprehensive expansion but provide insights into practice in different contexts.

Global rural health disparities in Alzheimer's disease and related dementias: State of the science.

INTRODUCTION: Individuals living in rural communities are at heightened risk for Alzheimer's disease and related dementias (ADRD), which parallels other persistent place-based health disparities. Identifying multiple potentially modifiable risk factors specific to rural areas that contribute to ADRD is an essential first step in understanding the complex interplay between various barriers and facilitators. METHODS: An interdisciplinary, international group of ADRD researchers convened to address the overarching question of: "What can be done to begin minimizing the rural health disparities that contribute uniquely to ADRD?" In this state of the science appraisal, we explore what is known about the biological, behavioral, sociocultural, and environmental influences on ADRD disparities in rural settings. RESULTS: A range of individual, interpersonal, and community factors were identified, including strengths of rural residents in facilitating healthy aging lifestyle interventions. DISCUSSION: A location dynamics model and ADRD-focused future directions are offered for guiding rural practitioners, researchers, and policymakers in mitigating rural disparities. HIGHLIGHTS: Rural residents face heightened Alzheimer's disease and related dementia (ADRD) risks and burdens due to health disparities. Defining the unique rural barriers and facilitators to cognitive health yields insight. The strengths and resilience of rural residents can mitigate ADRD-related challenges. A novel "location dynamics" model guides assessment of rural-specific ADRD issues.

A Policy Mapping Analysis of the U.S. Congressional Approach to Medical Aid-in-Dying.

This study examines the goals of medical aid-in-dying (MAID) legislation introduced to the US Congress from 1994-2020 using a policy mapping analysis approach. Using congress.gov, we identified 98 bills, 23 bills were analyzed in this study. Most of the bills aimed to restrict the use of federal funds, to regulate the drugs commonly used for MAID, to prohibit the development of policies or practices supporting MAID, and to regulate practitioners' roles in MAID. In practice, these bills would limit patient access to MAID by restricting drugs, funds, health care services, legal assistance, policy, and research. These findings suggest there lacks congressional support for MAID, even though polls of the public are divided yet favorable. Policymakers who support MAID should consider affirmative policies that 1) prevent MAID policies from discriminating against vulnerable groups, 2) support funding to study the use of MAID, and 3) build avenues to allow all qualified people to access MAID in places where it is legal.

Conversations about Community, Connection to Place, and Housing Preferences among Aging Adults in Lexington, Kentucky.

While many researchers have called for housing to be addressed in age-friendly cities and communities, little research exists to guide communities through this process. To maximize the potential for adults to age in place, researchers partnered with a local Age-Friendly community initiative to conduct focus groups and learn more about residents' preferences related to housing and residential space. The focus group data offered a glimpse into aging residents' perspectives on housing to inform planning and development of housing in a mid-size city. This second phase of a sequential mixed methods study included qualitative focus groups with 19 aging individuals. Focus group sessions sought to better understand previously identified housing preferences, aging adults' sense of community, and their connection to place. Findings suggest that aging participants cared not only about the physical structure of their home, but also the community-based aspects of where they live. Study implications suggest that social workers, working with and alongside community members and aging-service providers who have local knowledge, can support age-friendly housing models that consider the housing preferences of older residents, as well as the resources and limitations of their community.

Identifying Aging Adults' Housing Preferences: An Age-Friendly Initiative.

Community planning around housing needs of older adults is, at present, very understudied. This study stemmed from a practical need to determine the housing preferences of residents as part of an Age Friendly Community initiative. Data presented in this paper focus on the quantitative component of a sequential mixed methods study examining attitudes and preferences about housing. This first phase of this project involved data collected from 1,514 residents aged 30+ using a researcher-devised survey of 43 items. There was little difference in housing preferences between older and younger respondents or among those with varying financial means. Analysis revealed the most important housing considerations were safety in home, affordability, privacy, proximity to services frequently used, and accessibility. An unexpected finding was that almost half of older respondents expressed a willingness to share a home with a roommate. There were also differences in preferences on home environment based on current marital status. Data suggests that city planners and property developers should prioritize these preferences when planning for the housing-related needs of older residents. Future research should examine the interpretation of housing preferences, such as qualitative exploration of what it means for housing to be "safe" or 'affordable.'

Becoming a Caregiver: Experiences of Young Adults Moving into Family Caregiving Roles.

While the vast majority of family caregivers struggle to find balance between different roles in their lives, young adult caregivers are faced with the atypical challenge of caring for family member while simultaneously accomplishing developmental tasks typical of this stage in life (e.g., establishing career, developing romantic relationships). This exploratory, qualitative study examined strategies used by young adults to adopt family caregiving roles. These strategies can be described as embracement, compromise, and integration. While each approach allowed for the young adult to facilitate their caregiving role, additional research is needed to understand how the strategy affects the emerging adult's development.

Comparison of behaviors characteristic of autism spectrum disorder behaviors and behavioral and psychiatric symptoms of dementia.

BACKGROUND: Similarities exist in behavioral expression of autism spectrum disorder (ASD) and Alzheimer's disease and related dementias (ADRD). The purpose of this study was to assess presence of behavioral and psychiatric symptoms of dementia (BPSD) and ASD-like behaviors in adults with ADRD. METHODS: Using a cross-sectional design, data from University of Kentucky Alzheimer's Disease Center participant cohort were used. Hierarchical linear regression was used to assess (1) the relationship between ASD-like behaviors (measured by the Gilliam Autism Rating Scale-Second Edition, GARS-2) and BPSD measured by the Neuropsychiatric Inventory (NPI), and (2) the relationship between ASD-like behaviors and dementia severity (measured by the Clinical Dementia Rating [CDR] sum of boxes), when controlling for BPSD. RESULTS: Complete data were available for 142 participants. Using α of 0.05, analyses identified ASD behaviors were significantly associated with BPSD severity ratings (r = 0.47; p < 0.001) and dementia severity (r = 0.46; p < 0.001). GARS-2 explained 6.1% (p < 0.001) of variance in CDR sum of boxes when controlling for NPI and other covariates. DISCUSSION: There is significant overlap in behaviors characteristic of ASD and BPSD as assessed by the NPI and GARS-2, despite the use of these instruments in disparate developmental vs. aging settings. ASD behaviors appear to not be solely present in early childhood as a manifestation of ASD but are also present in older adults with neurodegenerative cognitive impairment. Such associations warrant additional research into causation, assessment, and behavioral interventions to further enable new therapeutic approaches targeting ASD behaviors across the lifespan.

Rural Older Adults in Disasters: A Study of Recovery From Hurricane Michael.

OBJECTIVE: This study aims for a greater understanding of how older adults (age 65 and older) in Jackson County, Florida, are prepared for and cope with the effects of a natural disaster. METHODS: A multidisciplinary, international research team developed a survey examining: (1) resources available to individuals aged 65+ in rural communities for preparing for a disaster; (2) challenges they face when experiencing a disaster; and (3) their physical, social, emotional, and financial needs when it strikes. The survey was administered with older adults (65+) in Jackson County, Florida, following Hurricane Michael in 2018. The descriptive, multivariate logistic, and linear regression analyses were performed to examine the relationship between respondents' demographic information and needs, concerns, and consequences of disaster. RESULTS: Results indicated (n = 139) rural community-dwelling older adults rely on social support, community organizations, and trusted disaster relief agencies to prepare for and recover from disaster-related events. CONCLUSIONS: Such findings can be used to inform the development of new interventions, programs, policies, practices, and tools for emergency management and social service agencies to improve disaster preparedness and resiliency among older populations in rural communities.

The Tele-STELLA protocol: Telehealth-based support for families living with later-stage Alzheimer's disease.

AIMS: We aim to establish the feasibility and acceptability of the Tele-STELLA (Support via Telehealth: Living and Learning with Advancing Alzheimer's Disease and Related Dementias) intervention. We will also assess the efficacy of the intervention in reducing the frequency of behavioural symptoms of dementia as well as family Care Partner reactivity to the symptoms. DESIGN: This is a multi-component, quasi-experimental study that focuses on facilitating effective management of behavioural symptoms that occur in the later stages of dementia. METHODS: Family Care Partners (n = 124) for persons with Alzheimer's disease will participate in two 8-week videoconferencing components that address behavioural symptoms-in both the persons with Alzheimer's disease and their Care Partners. In the first component ('Nova'), Care Partners work with one nurse for an hour/week for 4 weeks, then they join a small group for another 4 weeks. In the second component ('Constellation'), Care Partners work in a larger group to hone skills and knit supportive relationships. Behavioural symptom frequency and Care Partner reactivity to the behaviours will be measured prior to, during and after the intervention. The study is funded by the United States National Institute on Aging (R01AG067546); funding was initiated as on February, 2021. DISCUSSION: Tele-STELLA fills a gap in current videoconference-based psychoeducational interventions in that it offers real-time interaction with nurses and peers. The intervention was designed with feedback by pilot participants. This study will assess Tele-STELLA in its current, novel format; thus, preparing it for a larger, future randomized controlled trial. IMPACT: Tele-STELLA addresses symptoms that occur in the later stages of dementia, providing families with tools to facilitate effective behavioural management. Because Tele-STELLA is implemented via videoconferencing, it targets Care Partners who face barriers to support, such as cost and transportation. TRIAL REGISTRATION: This trial is registered with ClinicalTrials.gov (#NCT04627662).

Structural Characteristics of Nursing Homes and Social Service Directors that Influence Their Engagement in Disaster Preparedness Processes.

Nursing home residents are an at-risk population during disaster situations, and nursing homes face unique challenges in managing disasters. Nursing home social service departments can support their nursing homes in meeting the needs of residents during disasters, yet there is little research exploring their involvement. To address this gap, we use secondary data from the 2019 National Nursing Home Social Service Directors' study to explore social service directors' and their departments' involvement in disaster preparedness and response, and personal- and nursing home-level characteristics that predict involvement. Results show that nursing home social service directors and their staffs are predominantly involved; 61.9% (n = 562) of respondents stated always participating, and an additional 20.3% (n = 184) usually participating in disaster planning. The age of the director significantly predicted involvement, with older directors being most likely to always be involved. Further research is needed to understand why some nursing homes involve their social service directors in disaster planning and others do not, what roles those directors play, and to identify strategies to increase involvement within this role.

Older Adults' Adaptations to the Call for Social Distancing and Use of Technology: Insights From Socioemotional Selectivity Theory and Lived Experiences.

Social distancing guidelines during COVID can be isolating, especially for older adults, with potential for poor health outcomes. Technology offers opportunities for remote connection, yet, older adults' use of and perspectives on technology during this time remain largely unknown. The purpose of this study was to gain insight into older adults' technology use and preferences to inform the development of a technology training intervention to support older adult well-being. Semi-structured interviews were conducted with 30 older adults. Interviews were analyzed using an iterative, constant comparison approach. Findings were consistent with Socioemotional Selectivity Theory; respondents were primarily interested in technology to support emotionally meaningful goals. Participants indicated limited interest in technology training, referencing diminished future time perspectives to explain disinterest. Findings suggest that efforts to encourage older adults' expanded technology adoption should highlight how use supports emotionally meaningful goals and provide low-effort, timely training, tied to specific and clear applications.

Rural Caregivers: Identification of Informational Needs Through Telemedicine Questions.

OBJECTIVES: The prevalence of Alzheimer's disease and associated disorders is increasing. Rural residents in the United States have less access to memory care specialists and educational and community resources than in other areas of the country. Over a decade ago, we initiated an interdisciplinary rural caregiving telemedicine program to reach Kentucky residents in areas of the state where resources for supporting individuals with dementia are limited. Telemedicine programs involve a short informational presentation followed by a question and answer session; programs are offered 4 times a year. The purpose of this study was to explore questions asked over 1 year of the rural caregiving telemedicine program-encompassing 5 programs-to identify the scope of dementia-related knowledge gaps among attendees. METHODS: Questions from the 5 programs were recorded and content analyzed to identify areas of frequent informational requests. RESULTS: There were a total of 69 questions over the 5 sessions. For each program, questions ended due to time constraints rather than exhausting all inquiries. The most common topical areas of questions related to risk factors, behavioral management, diagnosis, and medications. DISCUSSION AND IMPLICATIONS: This study highlights that rural caregivers in Kentucky have diverse dementia educational needs. Rural communities may benefit from additional, targeted resources addressing these common areas of unmet informational needs.

Will "social distancing" lead to future "research distancing": A reflection on COVID-19 impacts on Alzheimer's disease research.

Coronavirus disease 19 (COVID-19) has dramatically altered everyday life, including the field of Alzheimer's disease (AD) research. This perspective article explores some of the ways in which COVID-19 has already impacted the field, anticipates some of the long-lasting effects, and explores strategies for addressing current and future needs. Areas of impact include study integrity, regulatory and industry issues, and participant engagement. Proposed strategies for addressing these challenges include analytic methods to deal with large degrees of missing data and development of patient-centered, user-friendly, remote data collection tools and assessments. We also highlight the importance of maintaining participant well-being as a first and constant priority.

Nature Prescriptions for Health: A Review of Evidence and Research Opportunities.

Nature prescription programs have emerged to address the high burden of chronic disease and increasingly sedentary and screen-based lifestyles. This study examines the base of evidence regarding such programs. We conducted a narrative review of published literature using four electronic databases. We included case studies, research design articles, and empirical studies that discussed any type of outdoor exposure or activities initiated by a health-care provider from an outpatient clinic. We examined articles for information on target populations, health outcomes, and structural and procedural elements. We also summarized evidence of the effectiveness of nature prescription programs, and discussed needs and challenges for both practice and research. Eleven studies, including eight empirical studies, have evaluated nature prescription programs with either structured or unstructured formats, referring patients either to nearby parks or to formal outdoor activity programs. Empirical studies evaluate a wide variety of health behaviors and outcomes among the most at-risk children and families. Research is too sparse to draw patterns in health outcome responses. Studies largely tested program structures to increase adherence, or patient follow-through, however findings were mixed. Three published studies explore providers' perspectives. More research is necessary to understand how to measure and increase patient adherence, short and long-term health outcomes for patients and their families, and determinants of provider participation and participation impacts on providers' own health.

Grief During the COVID-19 Pandemic: Considerations for Palliative Care Providers.

The COVID-19 pandemic is anticipated to continue spreading widely across the globe throughout 2020. To mitigate the devastating impact of COVID-19, social distancing and visitor restrictions in health care facilities have been widely implemented. Such policies and practices, along with the direct impact of the spread of COVID-19, complicate issues of grief that are relevant to medical providers. We describe the relationship of the COVID-19 pandemic to anticipatory grief, disenfranchised grief, and complicated grief for individuals, families, and their providers. Furthermore, we provide discussion regarding countering this grief through communication, advance care planning, and self-care practices. We provide resources for health care providers, in addition to calling on palliative care providers to consider their own role as a resource to other specialties during this public health emergency.

Considerations for Developing Online Bereavement Support Groups.

The loss of a family member or friend can have profound psychological and physical implications, particularly for individuals without bereavement support services. Online support groups can be an effective means of extending services beyond the traditional modes of delivery. This is especially true for populations that include isolated individuals and those with limited support networks, limited transportation, challenging time commitments, or reside in communities with limited services available. The literature over the last 10 years was reviewed to discern the potential opportunities and challenges of providing online bereavement support group services. Discussed are challenges for recruitment of participants, availability of technology resources, addressing privacy and confidentiality issues, participants' knowledge of technical equipment, legal considerations, ethical considerations, accessibility, and other best practices. Diverse populations such as adolescents, older adults, and rural communities must be uniquely considered when using online support groups.